NEOVAGINOPLASTY WITH NILE TILAPIA SKIN GRAFT IN A PATIENT WITH GONADAL DYSGENESIS: A CASE REPORT.

BACKGROUND: Gonadal dysgenesis, a genetic condition characterized by incomplete of defective formation of the gonads, can present with vaginal agenesis in individuals with 46,XY karyotype. CASE: We report an innovative intervention in the management of vaginal agenesis in a 19-year-old female with gonadal dysgenesis. Despite initial attempts with vaginal dilators, the patient presented unresponsive, leading to the adoption of a neovaginoplasty using Nile Tilapia Fish Skin (NTFS) as graft. The procedure, based on the McIndoe technique, involved the creation of a 10cm x 3cm vaginal canal with an NTFS-wrapped acrylic mold without complications. DISCUSSION: The use of NTFS as a graft for neovaginoplasty in gonadal dysgenesis, a novel approach not previously reported in medical literature for this diagnosis, demonstrated favorable outcomes in terms of functionality and patient well-being.

Illness experience and (unmet) needs of women with Mayer-Rokitansky-Küster-Hauser Syndrome (MRKH): a qualitative analysis of an online forum.

OBJECTIVE: Mayer-Rokitansky-Küster-Hauser syndrome (MRKH) is a congenital condition characterized by the underdevelopment or complete absence of the uterus and the upper part of the vagina. Diagnosis is commonly made during adolescence, a sensitive period for psychophysical development, following the absence of menstruation. Having MRKH syndrome can have a profound and multifaceted psychosocial impact that characterizes these women's subjective experiences, although it continues to be qualitatively understudied. This article explores the lived experience of women with MRKH spontaneously recounted on an online support forum. The aim was to gain insight into the features of the syndrome experience to understand deeply the emotional and social impact of the condition and the individual needs expressed online. METHODS: Using a naturalistic observation stance, data was collected from an online support forum for MRKH women and systematically analyzed using thematic analysis. RESULTS: Four main interconnected themes are identified: the impact of being diagnosed with MRKH, the difficult interaction with the medical environment, challenging social relationships, and the unmet needs of MRKH women. CONCLUSION: A multidisciplinary and person-centered approach that provides effective and sensitive management of the condition and its psychosocial implications, is essential. Recommendations for future research and practical clinical implications for healthcare professionals are proposed.

Long-Term Outcomes of the Restoration of Uterovaginal Continuity and Vaginoplasty-Utero-Colo-Neovaginoplasty-in Cervicovaginal Agenesis Using the Sigmoid Colon.

INTRODUCTION AND HYPOTHESIS: Congenital cervicovaginal agenesis in the presence of a functional endometrium is a rare Müllerian anomaly. The management ranges from hysterectomy historically to various reconstructive procedures more recently. We report our experience with utero-colo-vaginoplasty in the management of this anomaly and its long-term follow-up. METHODS: The case records of all the patients with vaginal or cervicovaginal agenesis in our hospital from January 2002 to December 2019 were reviewed retrospectively. The patients were then called for an outpatient visit and examined in detail. The anatomical variations, surgical procedures and outcomes were recorded and analysed. RESULTS: Sixteen patients aged 14 to 26 years were included during the study period. They presented with cyclical painful cryptomenorrhea. Magnetic resonance imaging (MRI) confirmed cervicovaginal or distal vaginal agenesis. All the patients underwent utero-colo-vaginoplasty. Intraoperative rectal injury led to post-operative faecal leak from the perineal wound in one patient in the post-operative period. Restoration of painless menstrual flow was possible in all 16 cases. Long-term complications were seen in 4 patients. These were stenosis of the perineal neovaginal orifice in 2 patients, obstruction at colo-uterine anastomosis in 1 patient and mucosal prolapse at the neovagina in 1 patient. Three of these patients needed secondary surgical procedures. Five were sexually active and reported consummation of penetrative intercourse. None of them had conceived. CONCLUSION: In our experience, utero-colo-vaginoplasty allows for regular painless menstruation and coitus with minimal long-term complications. The sole disadvantage is the failure to conceive.

Management of congenital cervical atresia with vaginal aplasia: A case report and review of the literature.

INTRODUCTION: Vaginal agenesis is a rare congenital condition, with an incidence of 1 in 4500 female births. CASE REPORT: We present a clinical case of vaginal aplasia with cervical atresia in a 31-year-old woman with primary amenorrhea. We aim to report the diagnostic process and provide a comprehensive outline of different possible treatments. DISCUSSION: The most common etiology of these agenesis cases is Mayer-Rokitansky-Küster-Hauser syndrome associated with uterine aplasia. However, vaginal aplasia can occur in 9 % of cases where the uterus is present. During embryogenesis, the Müllerian ducts give rise to the fallopian tubes, uterus, and upper two-thirds of the vagina, while the lower portion of the vagina develops from the urogenital sinus. Vaginal aplasia arises from a failure in the development of the terminal portion of the paramesonephric ducts. Abdominal pain, especially periodic pain, is the most common symptom, followed by primary amenorrhea. MRI is considered the gold standard for the diagnosis and precise description of female genital tract anomalies. CONCLUSION: Total hysterectomy remains a preferred option for cases of complete vaginal atresia to mitigate the risk of cervical or vaginal stenosis, adhesions, and pelvic inflammation resulting from poor menstrual blood drainage.

Experiences of pelvic and generalized persistent pain syndromes in MRKH: a scoping review.

STUDY OBJECTIVES: Mayer-Rokitansky-Küster-Hauser Syndrome (MRKH) is characterized by the congenital absence of the uterus and vagina, sometimes with associated extragenital anomalies. Currently there is limited literature on pelvic pain and co-morbid pain syndromes in people with MRKH. The aims of this scoping review were to summarize existing literature on pelvic and generalized persistent pain syndromes associated with MRKH and to identify knowledge gaps for further research into this field. METHODS: This scoping review followed the Joanna Briggs Institute framework. The population of interest was patients with a diagnosis of MRKH. MEDLINE, CINAHL, Scopus, Cochrane, Embase and Emcare databases were searched. Excluded were articles that did not meet the inclusion criteria or critical appraisal standards. The resultant articles were reviewed by 2 independent researchers while a third was used in cases of disagreement. A descriptive analytical method was used for data analysis. RESULTS: We screened 3348 articles for eligibility, 39 articles met criteria, which described 1353 cases of MRKH. 4 studies described baseline pelvic pain in MRKH, 19 described acute presentations and 13 described post-intervention pain levels. CONCLUSION: Despite the paucity of research, this review found that cyclical pelvic pain was mostly present in women with uterine remnants, whilst pelvic pain in those without remnants was poorly understood. There were no studies exploring generalized persistent pain syndromes in MRKH. Further cross-sectional studies are needed to elucidate prevalence and levels of pain syndromes in MRKH.

Uterus transplantation: A scoping review focused on obstetric outcomes.

BACKGROUND: Congenital uterine anomalies include a wide diversity of uterine malformations that can compromise reproductive potential. Uterus transplantation (UTx) proposes an innovative treatment for absolute uterine factor infertility; however, there is a lack of standardized protocols to guide clinical management among this population. OBJECTIVES: To describe recipient and donor characteristics and obstetric outcomes in patients undergoing UTx. SEARCH STRATEGY: We performed a literature search using the PubMed database to retrieve available scientific articles. We analyzed the references of included articles to assess additional articles that could be eligible to be included in the review. Likewise, we identified further studies using other methods, including Google Scholar. SELECTION CRITERIA: Titles and abstracts were screened in duplicate to select original reports with information available for the outcomes of interest. DATA COLLECTION AND ANALYSIS: This review assessed the advantages and disadvantages of the techniques used, patient characteristics, obstetric and non-obstetric complications, functional duration of the organ, and neonatal outcomes. MAIN RESULTS: Among the 36 reports included in this review we found 55 pregnancies and 38 live births following UTx and a higher success rate for in vivo uterine donations. The most common obstetric complications reported included miscarriage, pre-eclampsia, and gestational hypertension. The most common non-obstetric complications reported include episodes of rejection, acute kidney injury, anemia, and cholestasis. Living donors required a comprehensive preoperative workup, decreasing organ rejection, infection, and vascular complications. CONCLUSIONS: More studies are needed to standardize the UTx procedure and improve obstetric, fetal, and neonatal outcomes. Further understanding of which recipient and donor characteristics minimize complications will significantly decrease the risk of adverse outcomes.

Experiences of vaginal lengthening treatment and sexual well-being in women with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome: An interview study.

OBJECTIVE: To explore how women with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome experience dilation or surgical vaginal lengthening treatment, and their current sexual well-being. DESIGN: A qualitative interview study. SETTING: Denmark. POPULATION: Women aged ≥25 years diagnosed with MRKH syndrome. METHODS: Semi-structured video interviews were conducted with 18 women. Interviews lasted a median of 92 min and were digitally recorded, transcribed and anonymised. Data were analysed using thematic analysis. MAIN OUTCOME MEASURES: A qualitative analysis of women's experiences. RESULTS: The analysis identified three themes. Firstly, Experiences with dilation treatment revealed dilation as an awkward routine, especially for adolescents living with parents and yet to sexually debut. While some experienced successful vaginal lengthening, others faced treatment failure leading to frustration and self-blame. Secondly, Experiences with neovaginal surgery described the procedure as extremely painful but resulting in a 'normal size' vagina. Some women felt that the procedure had negatively impacted their self-confidence, and all underscored the importance of maturity before opting for surgery. Lastly, Current sex life and sexual well-being indicated a well-functioning sex life for many women, but with reported low sexual confidence and genital self-image due to the perceived 'deviance' of their genitalia. CONCLUSIONS: For women with MRKH syndrome, vaginal lengthening treatment, whether through dilation or surgery, may result in a 'normal size' vagina. However, according to the women's experiences, vaginal lengthening treatment does not adequately foster positive sexual esteem and genital self-image.

Rectovaginal fistula as a complication of rectal injury during vaginal reconstructive surgery: A case report.

INTRODUCTION AND IMPORTANCE: Rectovaginal fistula is a complication that may occur due to rectal injury during vaginal reconstructive surgery. To prevent these complications, the recognition of the injury is an important factor so that primary repair can be done. The primary repair can reduce the risk of complications such as fistula formation, and also reduce the physical and psychological impact on the patient. CASE PRESENTATION: A 33-year-old woman, came with a chief complaint of fecal leakage from the vagina and abdominal pain three months before admission with a history of vaginal reconstructive surgery due to vaginal agenesis. Eleven years after the reconstruction, the patient was diagnosed with recurrent obstruction caused by vaginal synechia. During the surgery of synechia release, rectum injury occurred. Even though primary closure repair was done at that time, several months later there was a complication of rectovaginal fistule formation in the form of fecal leakage from the vagina. The corrective surgery is performed in collaboration with a surgical gastroenterologist. CLINICAL DISCUSSION: Iatrogenic rectal injury may occur during gynecological surgery. A fistula that occurs after the reconstruction of vaginal agenesis is a high-type rectovaginal fistula, making the repairs more complex. Collaboration surgery between surgical gastroenterologist and gynecologist may be an option in such cases. CONCLUSION: Rectovaginal fistula is a rare but serious complication of vaginal reconstructive surgery. Early recognition, immediate management, and postoperative follow-up are essential in cases of rectal injury during vaginal reconstructive surgery.

Congenital cervico-vaginal aplasia with a functional uterus: A case report and review of literature.

Maldevelopment of the vagina and cervix is frequently accompanied by uterine aplasia or hypoplasia. Complete cervico-vaginal aplasia with a normally developing uterus is a very uncommon type of developmental failure. Failure to treat the condition can result in complications such as hematometra and hematosalpinx caused by the retrograde flow of blood into the fallopian tubes. In this case report, we describe the case of a 32-year-old woman experiencing cyclic abdominal pain and primary amenorrhea. The patient exhibited cervico-vaginal agenesis, with a functional uterus that was complicated by hematometra and bilateral hematosalpinx.

Histologic Analysis of 'Distraction Vaginogenesis' in a Rat Model.

Vaginal agenesis (VA) is frequently associated with mullerian agenesis. VA treatments include mechanical dilation and surgical vaginoplasty. We created a vaginal expansion sleeve (VES) as a novel device to progressively lengthen the vaginal canal. This study evaluated the histologic effects of the VES on rat vaginal tissue. The VES is a spring-like device made of proprietary woven cylindrical material and flat resin caps. The VESs were constructed as 25-30 mm, pre-contracted springs, which were secured into the vaginas of six Sprague Dawley rats and allowed to re-expand post-surgically. After one week, the VESs were removed, and the vaginas were harvested and measured in length. Test (n = 6) and control (n = 4) formalin-fixed paraffin-embedded tissues were stained with hematoxylin and eosin (H&E), Masson's trichrome, and anti-Desmin antibodies. The VESs achieved significant vaginal lengthening. The mean vaginal canal length increased from 20.0 ± 2.4 mm to 23.8 ± 1.2 mm after removal of the VESs (n = 6, p < 0.001), a 19% increase. There was a positive correlation between the expander/tension generated in the vagina and the amount of acute and chronic inflammation. H&E staining revealed increased submucosal eosinophilia in five of the six test tissues. One VES sample that was lengthened to 30 mm long showed evidence of lymphocytic and neutrophilic inflammation. Desmin immunostaining and Masson's trichrome stain revealed a thinner muscularis with more infiltrative fibrous tissue between muscle fibers in the test tissue compared to the control tissue. Although effective, the VES may provoke at least a transient increase in eosinophils consistent with a localized immune reaction during muscularis remodeling.

Satisfactory Outcome of Various Novel Modified Laparoscopic Neovaginoplasty Using Autologous Peritoneal Graft.

Management of vaginal agenesis in Mayer-Rokitansky-Küster-Hauser syndrome patients is by creating functional neovagina through surgical or nonsurgical route. Surgical repair using minimally invasive technique is a favorable option in creating neovagina. In this study, the patients underwent neovaginoplasty. Clinical follow-ups were done at 3, 6, and 12 months postoperatively. The primary outcomes were anatomic and functional successes; anatomical success was defined as a ≥6 cm-long neovagina that allows for easy introduction of two fingers, and functional success was defined with Female Sexual Function Index FSFI-6 questionnaire score above 19. Modified neovaginoplasty using autologous peritoneal graft was performed on the patients (n = 6). Follow-up showed mean vaginal lengths of 8.16 cm, mean surgery time of 175 min, mean blood loss of 59.17 ml, and mean duration of hospital stay of 2 days, with an average FSFI-6 score of 25,2. Therefore, we concluded that laparoscopic approach using modified technique of autologous peritoneal graft provides satisfactory result.

Understanding the Diagnostic Odyssey of Women with Mayer-Rokitansky-Küster-Hauser (MRKH) Syndrome in Denmark: A Qualitative Interview Study.

STUDY OBJECTIVE: The diagnosis of Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is often a lengthy process that typically occurs during late adolescence. To support optimized and patient-centered care, this study aimed to investigate how women with MRKH syndrome experience the diagnostic process. METHODS: From January 2021 to March 2021, we conducted in-depth interviews with 18 Danish women (≥25 years) diagnosed with MRKH syndrome. The interviews lasted a median of 92 minutes (range: 67-117). Data were analyzed using thematic analysis. RESULTS: As teenagers or young women at the time, all women had experienced the diagnostic process in the nonspecialized healthcare sector as deeply upsetting due to distressing gynecological examinations, use of inappropriate language, and considerable diagnostic delay. When reaching the specialized health care sector, questions could finally be answered, but this information and support did not significantly alter their feelings of being "deviant" or "flawed". The women continued their diagnostic odyssey beyond the health care system and found online communities that gave them valuable support in living with MRKH syndrome. CONCLUSION: Women experience the diagnostic odyssey of MRKH syndrome as upsetting and potentially traumatizing beyond the diagnosis. Healthcare professionals can influence young women's understanding and experience of MRKH syndrome by using inclusive language (eg, avoiding "deformity") and addressing all that is normal and functioning (eg, external genitalia and potential for sexual pleasure). In nonurgent conditions, young women should be given the choice to delay a genital examination.

Histological Features of Neovaginal Epithelium after Vaginoplasty in Mayer-Rokitansky-Küster-Hauser Syndrome.

OBJECTIVE: To analyze the features of the epithelia coating neovaginas after vaginoplasty in women affected by Mayer-Rokitansky-Küster-Hauser syndrome STUDY DESIGN: We conducted a retrospective analysis of prospectively collected data. Women affected by Rokitansky syndrome who underwent neovaginal biopsy after vaginoplasty (McIndoe surgery, intestinal vaginoplasty, Vecchietti surgery, and Davydov surgery) were included. Macroscopic mucosal features were assessed through clinical examination and the Schilling test. Each biopsy specimen was prepared for examination by light microscopy and in some cases by scanning electron microscopy (SEM). RESULTS: Thirty-six patients (4 McIndoe, 2 intestinal vaginoplasty, 14 Vecchietti, and 16 Davydov) were included. All biopsies were performed without complications. In McIndoe's neovaginas, the mucosal microscopic features were similar to normal skin, with large areas of preserved epithelium, heterogeneous presence of dermal papillae, and superficial keratinization. The characteristics of the intestinal neovagina's surface were similar to those of a sigmoid colon, with well-shaped glands, cylindrical cells, and a secreting mucosa. In Vecchietti neovaginas, the surface the epithelium was flat and multilayered, highly similar to that of a normal vagina, with the presence of glycogen and superficial desquamation. On medium SEM magnification evaluation, the epithelium presented flattened polygonal cells. Finally, in Davydov neovaginas, none of the specimens had persistent mesothelial elements. The squamous neo-epithelium had regular aspects of differentiation with the presence of glycogen. At greater SEM magnification, microridges were evident, with a regular distribution. CONCLUSION: Each different technique of vaginoplasty leads to unique histological and structural features of the neovagina's mucosa. Knowledge of these elements must be the basis for the choice of the most appropriate intervention.

Neovagina creation methods in Müllerian anomalies and risk of malignancy: insights from a systematic review.

PURPOSE: This systematic review aims to provide a data synthesis about the risk of neovaginal cancer in women with Müllerian anomalies and to investigate the association between the adopted reconstructive technique and the cancer histotype. METHODS: PubMed, MEDLINE, Embase, Scopus, ClinicalTrials.gov and Web of Science databases were searched from inception to March 1st, 2023. Studies were included if: (1) only women affected by Müllerian malformations were included, (2) the congenital defect and the vaginoplasty technique were clearly reported, (3) the type of malignancy was specified. RESULTS: Literature search yielded 18 cases of squamous cell carcinoma and two cases of vaginal intraepithelial neoplasia 3 (VAIN 3). Of these, 3 had been operated on according to the Wharton technique, 8 according to the McIndoe technique, 3 with a split-skin graft vaginoplasty, 2 according to the Davydov technique, 2 with a simple cleavage technique, 1 according to the Vecchietti technique and 1 with a bladder flap vaginoplasty. A total of 17 cases of adenocarcinoma and 1 case of high-grade polypoid dysplasia were also described. Of these, 15 had undergone intestinal vaginoplasty, 1 had been operated on according to the McIndoe technique and 1 had undergone non-surgical vaginoplasty. Finally, 1 case of verrucous carcinoma in a woman who had undergone a split-skin graft vaginoplasty, was reported. CONCLUSION: Although rare, neovaginal carcinoma is a definite risk after vaginal reconstruction, regardless of the adopted technique. Gynaecologic visits including the speculum examination, the HPV DNA and/or the Pap smear tests should be scheduled on an annual basis.

Müllerian Agenesis in a patient with Rubinstein-Taybi Syndrome: A Case Series and Review of the Overlapping Developmental Biologic Pathways.

BACKGROUND: Rubinstein-Taybi syndrome (RSTS) is a multi-system neurodevelopmental condition caused by deficiency of CREBBP (16p13.3) or EP300 (22q13.2). Müllerian agenesis, or Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, is defined as congenital agenesis of the uterus, cervix, and upper vagina without a definite genetic cause. INDEX CASE AND CASE SERIES: We present a 14-year-old female with RSTS type 1 (CREBBP, c.4395-2A>C) and MRKH, the first documented in the literature. Following presentation to Gynecology for anticipatory guidance regarding future menstrual suppression and follow-up of previously diagnosed labial adhesions, exam under anesthesia revealed a single urogenital opening with cystoscopy demonstrating a normal urethra and bladder. Laboratory evaluation was consistent with peripubertal female gonadotropins and estradiol, 46,XX karyotype, and normal microarray, and a pelvic MRI confirmed Müllerian agenesis. Given this case, we assessed our cohort of females with RSTS and found that 4 of 12 individuals also had Müllerian anomalies. CONCLUSION: Gynecologic evaluation should be a part of medical care for females with RSTS, particularly in individuals with delayed menarche or abnormal menstrual history, on the basis of the observed association between RSTS and Müllerian anomalies in this case series. Although several candidate genes and copy number variants are associated with MRKH, no candidate genes in close proximity to the 16p13.3 region have been identified to explain both RSTS and MRKH in the index patient. Due to the regulatory nature of CREBBP during embryonic development, we theorize that CREBBP may play a role in the migration of Müllerian structures during embryogenesis.

Long-Term Bowel Vaginoplasty With a Single Yang-Monti Tube.

INTRODUCTION: The bowel remains the favored tissue for neo-vaginoplasty (NeoVP) in pediatric patients with vaginal agenesis. In 2001, the first description of NeoVP using the Yang-Monti technique with a sigmoid double tubular flap was published. We present our experience of NeoVP with a single Yang-Monti tube (SYMT) flap and report on the use of different segments of colon. METHODS: We conducted a retrospective review of patients who underwent NeoVP using a bowel SYMT between 2009 and 2021. The procedure was performed under open abdominal surgery by isolating 8 to 12 cm from the rectosigmoid, cecum, or ascending colon. Subsequently, this segment was detubularized near the mesenteric border, folded, and retubularized transversally, leaving the mesentery in a cephalic position. A channel was dissected in the pelvis to accommodate the NeoVP. RESULTS: Seventeen patients were identified. The median age was 16 years old. The principal diagnosis was Mayer-Rokitansky's syndrome (47.1%). The median follow-up was 50 months. The mean harvested bowel length was 9 cm, and the sigmoid was the preferred site (65%). The cecum-ascending colon was used in 3 (17.6%) patients. Complications were recorded in 6 patients (35%). Of these 6 patients, 4 had introital stenosis. There was 1 case of urethrovaginal fistula and another of left hematometrocolpos. Satisfactory sexual function has been reported in sexually active individuals. CONCLUSIONS: We described our experience in NeoVP using a large bowel SYMT as a safe and effective technique. It allows decreased tension on the vascular pedicle and the use of shorter colon segments.

Effectiveness of non-surgical interventions to improve health and well-being in women living with Mayer-Rokitansky-Kuster-Hauser syndrome: A systematic review.

AIM: The aim of this paper is to present the evidence on the effectiveness of non-surgical interventions to improve health and well-being in women living with Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome. DESIGN: Systematic review guided by Preferred Reporting Items for Systematic Reviews checklist. DATA SOURCES: The search was conducted between June and September 2022 across the following databases: CINAHL, EMBASE, Medline, PsycINFO and Cochrane. Trial registries (clinicaltrials. gov, World Health Organization International Clinical Trials Registry Platform (ICTRP), Cochrane Controlled Trials Register-CCTR), Google scholar, dissertations, conference proceedings and reference lists of included studies were also searched. Corresponding authors, formal and informal MRKH groups were contacted to obtain any significant studies or reviews. REVIEW METHODS: Eligible were only English-language empirical studies of any time period. The review followed narrative synthesis. RESULTS: Twenty-three studies were identified that fit the inclusion criteria which included 1540 MRKH syndrome affected women. Four studies were on psychological interventions (n = 85) and 19 studies (vaginal dilation therapy n = 897, coital dilation n = 57) focused on non-surgical vaginal dilation as a measure to vaginal agenesis in MRKH syndrome. CONCLUSIONS: Clearly, vaginal dilation is a viable initial treatment option for women with MRKH syndrome. There is limited evidence that 'coital dilation' is an effective method of dilation for vaginal agenesis. The literature, however, supports the need for psychological intervention to improve health and well-being. IMPACT: Women with MRKH syndrome who require dilation can receive guidance and support from their healthcare providers, particularly sexual and reproductive health nurses, clinical nurse specialists and gynaecologists. From the point of diagnosis, clinical psychologists should be involved. As much as feasible, family and partner support can be encouraged. PATIENT OR PUBLIC CONTRIBUTION: No patient or public contribution.

Nonsurgical neovagina creation in congenital vaginal agenesis: a case report of movement-based dilator therapy.

Objective: To report the use of progressive, high-frequency movement-based dilator therapy (MBDT) to create a neovagina in a patient with congenital vaginal agenesis. Design: Case report. Setting: Tertiary care military hospital. Patients: A 22-year-old woman with congenital vaginal agenesis. Interventions: Self-directed MBDT. Main Outcome Measures: Vaginal elongation by self-directed MBDT. Results: The patient achieved a 6.5-cm vaginal length after 6 pelvic health physical therapy sessions over a span of 4 months of progressive, high-frequency MBDT. Conclusions: Progressive, high-frequency MBDT should be considered as part of a first-line dilator therapy regimen for patients with congenital vaginal agenesis interested in creating a neovagina.

Surgical Correction of Vaginal Agenesis via Modified Laparoscopic Vecchietti Procedure.

OBJECTIVE: To describe surgical correction of vaginal agenesis via a modified laparoscopic Vecchietti procedure with the goal of disseminating knowledge and improving surgical technique CASE: An 18-year-old female presented with primary amenorrhea, age-appropriate secondary sex characteristics, a shallow vagina, and 46,XX karyotype. Imaging showed rudimentary uterine horns and normal ovaries, kidneys, and spine. Diagnosis of Mayer-Rokitansky-Küster-Hauser syndrome type I was made. After an unsuccessful attempt at vaginal dilation and extensive counseling, the patient chose to have a laparoscopic Vecchietti procedure. Vecchietti vaginoplasty eliminates the need for grafts and creates a neovagina with accelerated vaginal dilation by stretching the introital mucosa with a spring mechanism. RESULTS: A modified laparoscopic Vecchietti procedure was performed. Postoperatively, daily suture adjustments were made. When the device was removed after 7 days, the examination revealed a 9-cm vaginal canal, which was maintained with self-dilation.